cLFM-Qol: A specific quality of life measurement tool for children from 11 to 15 years with low-flow malformations.

BACKGROUND: Low-flow malformations (LFMs) are rare diseases with a significant impact on health-related quality of life (HRQoL), especially in children. No disease-specific questionnaire is available for children with LFMs. OBJECTIVE: To develop and validate a specific HRQoL questionnaire for children from 11 to 15 years old suffering from LFMs. METHODS: A preliminary questionnaire based on a verbatim from focus groups was created and sent to children from 11 to 15 years old suffering from LFMs, together with a dermatology-specific and a generic HRQoL questionnaire (cDLQI and EQ-5D-Y). RESULTS: A total of 75 from 201 included children responded to the questionnaires. The final version of the questionnaire (cLFM-QoL) included 15 questions and was not divisible into subscales. It demonstrated excellent internal consistency (cronbach 0.89), convergent validity and readability (SMOG 6.04). cLFM-QoL mean score (± SD) was 12.9/45 (8.03) for all grades of severity, for mild 8.22/45 (7.5), moderate 14.03/45 (8.35), severe 12.35/45 (6.59) or very severe patients 20.7/45 (3.39) (p 0.006). CONCLUSION: cLFM-QoL is a validated short and easy to use specific questionnaire with excellent psychometric capacities. It will be suitable for any children aged 11-15 with LFMs, in daily practice or clinical trials.

Severe Vulvar and Perianal Crohn's Disease: A Case Report and Review of the Literature.

Association of vulvar Crohn's disease (CD) with pyoderma gangrenosum (PG) has been described, yet due to its low prevalence, data on therapeutic management of this association are still limited. We here present a 22-year-old woman with severe vulvar and perianal CD with a major inguinal and perineal ulceration. In the hypothesis of an associated PG, oral corticosteroids were prescribed at a dose of 40 mg with limited efficacy for a duration of 3 months. Afterward, a combination treatment of cyclosporine and infliximab followed by negative pressure wound therapy and split-thickness skin graft resulted in complete healing.

Are topical beta-blockers really effective "in real life" for targeted therapy-induced paronychia.

Paronychia and periungual pyogenic granuloma represent one of the most common and bothersome dermatologic toxicities observed with ErbB inhibitors. There is no standardized treatment, and management remains challenging. Moreover, conservative management with noninvasive treatment should be promoted for fragile patients in a metastatic setting. Over the last few years, the efficacy of topical blocking agents has been considered for managing cutaneous or mucosal pyogenic granulomas. Very recently, the use of topical propranolol or of timolol has been reported in several patients undergoing treatment with EGFR inhibitors and developing pyogenic granulomas of the nail. We performed a retrospective single-center review of patients with targeted therapy-related paronychia/periungual pyogenic granulomas who had been treated with topical timolol, either alone or in combination with other topical treatments. Nearly two thirds of patients showed at least a partial response after 1 month of therapy, and the use of a topical beta-blocker in our population was associated with a favorable safety profile. Finally, topical timolol may represent a promising treatment option for the management of cancer patients suffering from painful periungual lesions. Comparative clinical trials, however, are still needed.

Enzalutamide induced acute generalized exanthematous pustulosis.

INTRODUCTION: Enzalutamide (Xtandi®) is a new potent inhibitor of the signaling pathway for the androgen receptor with a half-life of 5.8 days. It has been on the market for the treatment of metastatic castration-resistant prostate cancer since November 2013. OBJECTIVE: We report the first case of acute generalized exanthematous pustulosis (AGEP) induced by enzalutamide. OBSERVATION: A 62-year-old male patient with no significant medical history, was diagnosed in April 2014 with metastatic prostatic adenocarcinoma. In April 2015 the patient received a second line oral therapy with enzalutamide, 160 mg/day, coupled with a subcutaneous implant of 10.8 mg of goserelin, an agonist analog of natural luteinising hormone releasing hormone (LH-RH). Ten days after starting enzalutamide treatment and four days after introduction of first goserelin subcutaneous implant, the patient experienced an acute skin reaction. It is about of the plaques covered with widespread millimetric non-follicular pustules. Complete resolution of skin lesions occurred within four weeks. According to the AGEP validation score of the European Study of Severe Cutaneous Adverse Reactions, the total score in the current case was 7, interpreted as probable AGEP. According to criteria that assess adverse drug reactions, it was concluded that enzalutamide was responsible for this case of AGEP (suggestive imputation). CONCLUSIONS: Dermatologist can be confronted with adverse skin drug reactions attributable to new therapeutic molecules. The slow resolution of symptoms seems be due to the long half-life of enzalutamide.